Hepato-pancreato-biliary Abnormalities in Fibrous Dysplasia of Bone/McCune Albright Syndrome

Recruiting

• Conditions: Fibrous Dysplasia of Bone

• Registration Number: NCT06177327
• Lead Sponsor: Hospices Civils de Lyon

Brief Summary

Fibrous dysplasia of bone /McCune Albright syndrome (FD/MAS) is a rare bone disease caused by somatic mutations in GNAS gene. This GNAS mutation predisposes to cancers, including breast cancer, thyroid cancer, chondrosarcoma and osteosarcoma, as well as biliary tract anomalies, liver-tumors or pancreatic tumors - IPMNs. Intraductal papillary and mucinous neoplasms of the pancreas (IPMN) are cystic intraepithelial ductal lesions developed at the expense of pancreatic ducts. They are pre-cancerous lesions, requiring monitoring and, in case of progression or malignant degeneration, surgical resection. Pancreatic MRI screening of patients with polyostotic FD and MAS is recommended.

The aim of this study is to investigate the epidemiology and characteristics of these hepato-pancreato-biliary abnormalities (prevalence, age of onset, degeneration), based on magnetic resonance imaging (MRI) realized during the follow-up of patients with FD/MAS treated in a French FD expert center.

A better understanding of these IPMNs and other digestive abnormalities will enable clinicians to improve the management and monitoring in this high-risk population.

Detailed Description

Not available

Study Timeline

• Study First Submitted: 2023-12-11
• Study First Submitted QC: 2023-12-11
• Study First Posted: 2023-12-20
• Study Start: 2024-01-19
• Status Verified: 2024-02
• Last Update Submitted: 2024-02-27
• Last Update Posted: 2024-02-29
• Primary Completion: 2024-11-30
• Study Completion: 2025-12-12

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 545

Inclusion Criteria

• Adults ≥18 years with polyostotic FD/MAS
• Followed up in the national FD reference center (Lyon) or in a FD French expert center: Angers, Bordeaux, Brest, Caen, Clermont-Ferrand, Lille, Marseille, Montpellier, Nice, Paris, Poitiers, Rennes, Saint-Etienne, Strasbourg.
• who had an hepato-bilio-pancreatic MRI during their follow-up, since January 2002
• No objection to data collection

Exclusion Criteria

-Opposition to data collection

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Describe hepato-bilio-pancreatic abnormalities in FD/MAS and estimate the prevalence of IPMN.	hepato-bilio-pancreatic MRI performed from January 2002 until December 2024.	Hepato-bilio-pancreatic abnormalities (size, number, complications) will be described based on hepato-bilio-pancreatic MRI.

Trial Locations

Locations (16)

CHU Angers; 🇫🇷 Angers, France

Groupe Hospitalier Pellegrin - Chu; 🇫🇷 Bordeaux, France

CHU de Brest - Hôpital de la Cavale Blanche; 🇫🇷 Brest, France

Chu de Caen Normandie; 🇫🇷 Caen, France

Chu de Clermont-Ferrand; 🇫🇷 Clermont-Ferrand, France

Chu de Lille; 🇫🇷 Lille, France

Service Rhumatologie, pavillon F Hopital E. Herriot; 🇫🇷 Lyon, France

Assistance Publique - Hopitaux de Marseille (Ap-Hm; 🇫🇷 Marseille, France

Chu de Montpellier; 🇫🇷 Montpellier, France

CHU de Nice; 🇫🇷 Nice, France

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